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Cordenier et al.

The Journal of Headache and Pain 2013, 14:42


http://www.thejournalofheadacheandpain.com/content/14/1/42

REVIEW ARTICLE Open Access

Headache associated with cough: a review


Ann Cordenier1, Willem De Hertogh2, Jacques De Keyser1,3 and Jan Versijpt1,4*

Abstract
Headache only triggered by coughing is a rather uncommon condition. The aim of the present review is to present
an overview of the diagnosis, clinical characteristics, pathophysiology and treatment of both primary and
symptomatic cough headache and discuss other relevant headache disorders affected by coughing. The diagnosis
of primary cough headache is made when headache is brought on and occurs only in association with coughing,
straining or a Valsalva manoeuvre and in the absence of any abnormalities on neuro-imaging. In case an underlying
pathology is identified as a cause of the headache, the diagnosis of symptomatic cough headache is made. The
vast majority of these patients present with a Chiari malformation type I. Other frequently reported causes include
miscellaneous posterior fossa pathology, carotid or vertebrobasilar disease and cerebral aneurysms. Consequently,
diagnostic neuroimaging is key in the diagnosis of cough-related headache and guides treatment. Besides primary
and symptomatic cough headache, several other both primary and secondary headache disorders exist where
coughing acts as a trigger or aggravator of headache symptomatology.
Keywords: Cough, Headache, Diagnosis, Treatment

Review both primary and secondary headache disorders which are


Cough triggering headache is an uncommon finding. It is triggered or aggravated by coughing are described.
characterized as headache triggered by rapid increases in
intra-abdominal pressure, caused by coughing, sneezing
Methods
or straining. The life-time prevalence of cough headache
Search strategy and selection of articles
is estimated to be 1% [1]. The prevalence in a headache
The MEDLINE database was searched between 1950 up
clinic varies from 0.4% to 1.2% [2,3].
till 2011, using the MeSH terms “cough”, “headache”, and
Cough headache can be further subdivided into primary
“Valsava manoever”. The search was limited to English
and symptomatic cough headache. It has first been de-
studies in humans. Articles were included when dealing
scribed in medical literature in 1932 by Tinel [4]. Initially,
with diagnostics or therapy of cough-related headache in
cough headache was considered as an alarm symptom,
adults. Articles were excluded when ‘headache’ and ‘cough’
until both Symonds and Rooke reported cases of benign
where mentioned as symptoms of other medical condi-
cough headache, now known as primary cough headache
tions (e.g. cold, hypertension, non-specific health symp-
[5,6]. Up till now, about 400 cases of primary and 300
toms, related to surgical procedures such as stereotactic
cases of symptomatic cough headache have been de-
surgery, or related to substances). Case series and single
scribed in literature. In addition to previous reviews on
cases were included. Bibliographies of selected articles
primary cough headache [7], we review the etiology, clin-
were screened for additional relevant articles.
ical features and treatment strategies for both primary as
well as symptomatic cough headache and shed light on
some pathophysiological mechanisms. Moreover, other Cough headache
Primary cough headache
In 1956, Symonds was the first to describe primary cough
* Correspondence: jan.versijpt@uzbrussel.be headache as a separate disease entity. He described 27
1
Headache Clinic, Department of Neurology, Universitair Ziekenhuis Brussel, cases of headache provoked by Valsalva maneuvers like
Brussel, Belgium coughing, sneezing, straining, laughing or stooping [5]. In
4
Department of Neurology, Universitair Ziekenhuis Brussel, Laarbeeklaan 101,
1090, Brussel, Belgium 21 of these patients, no intracranial lesion by means of
Full list of author information is available at the end of the article computed tomography could be demonstrated. Later, a
© 2013 Cordenier et al.; licensee Springer. This is an Open Access article distributed under the terms of the Creative Commons
Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction
in any medium, provided the original work is properly cited.
http://www.thejournalofheadacheandpain.com/content/14/1/42
Cordenier et al. The Journal of Headache and Pain 2013, 14:42
Table 1 Major characteristics of published series on primary cough headache
First author,
Nr of Cases Patient characteristics Headache characteristics
Year [Reference]
M/F Mean Associated
Intensity & type Other triggers Duration Location Frequency Persistence
ratio age features
Symonds, 1956 severe Valsalva
18 months-
21 18/3 55 maneuver, 2’-10’ bilateral - -
3 years
[5] bursting head rotation
moderate to
Pascual,1996 severe seconds to
Valsalva bilateral (92%) one to several
13 10/3 67 less than 2-24 months none
manoeuver unilateral (8%) daily
[13] sharp, 30’
stabbing
moderate to
Ozge, 2005 severe not mentioned
bilateral (90%) 10 nausea (5%)
20 13/7 45 or no other 1-30’ -
unilateral (10%) days/month dizziness (10%)
[9] sharp, triggers?
stabbing
electrical,
sudden postural unilateral (50%)
explosive,
Pascual, 2008 movements, seconds to bilateral (39%)
pressing or
28 10/18 60 weight lifting, more than occipito- - 1-42 months dizziness (14%)
having a
[11] laughing and 1’ suboccipital
mixed
defecating (11%)
nature
nausea (10%)
mild to
vomiting (1%)
Chen, 2009 severe
straining at photophobia
bilateral (67%)
74 54/20 61 stool and 1” - 2 hours - 6-24 months (5%)
[3] explosive, unilateral (33%)
bending down phonophobia
dull,
(11%)
pulsatile

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series of 93 patients with ‘benign exertional headache’ was photo- and phonophobia are uncommon [12]. Besides
described by Rooke in 1968. He did not make a difference cough, headache was also triggered by other Valsalva
between cough headache and headache provoked by phys- maneuvers in most of the studies, but never by physical
ical exercise [6]. exercise.
Primary cough headache, previously also called benign
cough headache [7] or Valsalva-manoeuvre headache, is Symptomatic cough headache
currently defined by the International Headache Society Underlying etiologies are present in approximately 40%
(IHS) as a headache, precipitated by coughing or of the cases and are mostly related to Chiari type I mal-
straining in the absence of any intracranial disorder last- formation. In general, patients with symptomatic cough
ing up to 30 minutes [8]. headache differ from patients with primary cough head-
ache in the fact that they tend to have more associated
Diagnostic criteria for primary cough headache (group symptoms, depending on the underlying abnormality.
4.2 - International classification of headache disorders, Additional headache triggers, higher pain intensities and
2nd edition, 2004) diverse headache durations and locations are generally
reported. The major causes of symptomatic cough head-
A. Headache fulfilling criteria B and C ache are shown in Figure 1. The most common causes
B. Sudden onset, lasting from one second to 30 minutes are, after Chiari type I malformation, miscellaneous pos-
C. Brought on by and occurring only in association terior fossa lesions. Other causes include obstructive
with coughing, straining and/or Valsalva manoeuvre hydrocephalus and spontaneous low cerebrospinal fluid
D. Not attributed to another disorder pressure (CSF).
All of the 17 patients (10 men) with symptomatic
Table 1 gives an overview of the published case series cough headache reported by Pascual et al. had a Chiari
of primary cough headache. type I malformation. In contrast to patients with primary
Primary cough headache is usually bilateral but can be cough headache, they all had occipital or suboccipital
unilateral and has a moderate to severe intensity where headache. Pain was described as bursting, stabbing, dull,
the type of pain varies. According to Özge et al., pain or lancinating. Headache durations ranged from seconds
was mostly located in the frontotemporal regions but to several weeks. Besides cough, headache could be pro-
even toothache as the presenting symptom has been de- voked by laughing, weight lifting or acute body or head
scribed [9,10]. It most often affects men, however, postural changes. All patients also developed one or
Pascual et al. reported on 28 patients with primary more posterior fossa symptoms, however, not all from
cough headache, of which 18 were women [11]. It usu- the start [13].
ally affects subjects over the age of 40. According to the More recently, Pascual et al. reported on forty patients
currently available criteria, the headache should last (12 men) with symptomatic cough headache. Thirty-two
from one second to 30 minutes, but headaches of a lon- patients had a Chiari type I malformation. Eight patients
ger lasting duration have been reported. For instance, had a structural lesion in the posterior fossa: 3 arach-
Chen et al. published a series of 74 primary cough head- noid cysts, 2 dermoid tumours, 2 meningiomas and 1 os
ache patients where the median headache duration was odontoideum. Headache was localized occipito-sub-
indeed 30 seconds, but in a minority of patients the occipital. The nature of the pain was described as press-
headache lasted up to 2 hours [3]. Nausea, vomiting, ing, explosive, electrical or having a mixed nature.
Duration ranged from seconds to more than one minute.
20% Postural movements, laughing and defecation could also
trigger the headache. Thirty-three patients had posterior
fossa symptomatology like dizziness, unsteadiness, facial
and upper limb numbness, vertigo and syncope [11].
Chen at al. reported on nine symptomatic cough head-
15% ache patients. The headache profile was similar as in their
65%
primary cough headache patient group being mostly bilat-
eral and rarely with associated symptoms. The duration of
Chiari malformation type I the headache ranged from 10 seconds to 30 minutes. In
this group, headache could be triggered not only by cough,
posterior fossa lesions but also by exertion, straining at stool and lifting heavy
other objects. As underlying causes they found 4 patients with
an obstructive hydrocephalus, two patients with a Chiari
Figure 1 Etiology of symptomatic cough headache.
malformation type I, one patient with a subdural
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hematoma, one with multiple brain metastases and one had a more crowded posterior cranial fossa. This might
with an acute sphenoid sinusitis [3]. lead to a relative obstruction of CSF flow, which can
Nuti et al. and Evans et al. both described a patient contribute to an increase in intracranial pressure during
who presented with cough headache due to spontaneous coughing [27].
low CSF pressure [14-16]. Mokri also described two pa- An increase in intracranial pressure is also believed to
tients with headache provoked by cough and Valsalva be the underlying mechanism of symptomatic cough
maneuvers caused by a spontaneous CSF leak [17]. One headache, although the exact mechanism is unknown. In
case of pneumocephalus and pneumococcal meningitis patients with a Chiari malformation type I, this seems to
presenting with cough headache was also reported [18]. be caused by the sagging of the cerebellar tonsils below
Eross et al. reported on a case of a 66-year old patient the foramen magnum [28,29]. Indeed, Williams de-
with cough headache where magnetic resonance imaging scribed two patients with cough headache and a tonsillar
revealed a posterior fossa mass, associated with obstruct- herniation where a difference in pressure between the
ive hydrocephalus [19]. Two case reports even mention ventricles and the lumbar subarachnoidal space after
cough headache as the presenting symptom of carotid performing a Valsalva manoever was demonstrated [28].
artery disease [20,21]. Finally, Smith and Messing report This craniospinal pressure dissociation displaces the ton-
on one case of cough headache associated with a non- sils further into the foramen magnum and pain by
ruptured cerebral aneurysm [22]. coughing could therefore be caused by compression or
tracking on pain-sensitive structures in the arachnoid
Pathophysiology space or blood vessels surrounding the tonsils. This
The pathophysiology of primary cough headache is not mechanism is supported by the fact that, after surgery,
well understood, but various hypotheses have been for- both the craniospinal pressure dissociation and cough
mulated. It seems likely that it is associated with an in- headache disappear. Moreover, Pascual et al. found that
creased intracranial pressure caused by coughing, this headache correlated with the degree of tonsillar descent
due to an increase in the intra-thoracic and intra- [30], although this was not supported by the findings of
abdominal pressure subsequently leading to an increase Sansur et al. They also did not find a craniospinal pres-
in the central venous pressure. A recent study showed a sure dissociation and postulated that headache was asso-
transverse or jugular vein stenosis by means of MR ven- ciated with a sudden increase of intracranial pressure,
ography in 5 out of 7 patients with primary cough head- caused by obstruction of free CSF flow in the subarach-
ache, although the debate continues as to whether this noid space [29].
stenosis is a primary or secondary process related to
raised intracranial pressure [23]. Patients might also Treatment
have a lower threshold for pain associated with the in- Although no long term studies exist on the natural evo-
crease in intracranial pressure caused by coughing [24]. lution of cough headache, it seems that most of the pri-
Raskin further hypothesized about the location of hyper- mary cough headache patients remit spontaneously after
sensitive pressure receptors on the venous vessel walls maximum 4 years, however, patients with a disease dur-
[24]. Wang on the other hand proposed that cough ation of 12 years and more have been described [5]. Be-
headache was caused by CSF hypervolemia, which would cause of the short duration, there is usually no need for
lead to an increase in intracranial pressure during an acute treatment. However, since symptoms can be
coughing [25]. Wolff thought that cough headache was quite debilitating, a preventive treatment strategy should
related to a systemic infection, which would alter the be considered in most if not all patients. Treatment op-
vascular tone in the cranial vessels [26]. Finally, Chen tions for primary cough headache are outlined in Table 2.
et al. found that patients with primary cough headache Apart from one small double-blind, placebo-controlled

Table 2 Reported treatments for primary cough headache


Product [Reference] Recommended daily dose Most common side effects
Indomethacin [3] 50-150 mg peptic ulcers, dyspepsia, edema, hyperkalemia, hypernatremia, hypertension
Topiramate [33] 50-100 mg cognitive deficits, paresthesia, anorexia
Methysergide [9,34,35] 2 mg pleuritis, pericarditis, retroperitoneal fibrosis
Acetazolamide [25] 375-2200 mg paresthesia, parageusia, kidney stones, dehydration, headache, metabolic acidosis
Propranolol [35] 120 mg hypotension, bradycardia
Naproxen [37] 550-1100 mg gastrointestinal complaints
Metoclopramide [38] 10 mg intravenous bolus restlessness, drowsiness, dizziness, fatigue, and focal dystonia
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crossover study with indomethacin in a dose of 50 mg Patients with symptomatic cough headache usually re-
tid, no large randomized trials have been performed quire a tailor-based surgical treatment. Suboccipital
[31]. General consensus exists that the treatment of craniectomy, whether or not combined with a C1-C3
choice for primary cough headache is indeed indometh- laminectomy, relieves cough headache in the majority of
acin, however with varying daily doses, treatment dura- patients with a Chiari malformation type I [11,13]. Of
tions and treatment effects with a general response rate interest is the fact that, although not consistently, a re-
of approximately 73% [3]. Several studies found that sponse rate to indomethacin of approximately 38% has
daily doses ranging from 25–150 mg usually are effect- been described in several symptomatic cough headache
ive [3,9,11,13,31]. In one study a daily dose up to patients [3,11,39].
250 mg was required [24]. In the series published by
Chen et al., less than half of the patients experienced a Other relevant headache disorders, potentially triggered
complete relief where another one third had a partial or aggravated by coughing
response [3]. No consensus exists on treatment dur- Next to primary and symptomatic headache, several
ation. In the series of Pascual et al., treatment was re- other both primary and secondary headache disorders
quired for a maximum period of 5 months and in the exist where coughing is a known trigger for headache
series published by Chen et al., nearly every patient symptomatology or where headache can be aggravated
with a good initial response was pain-free within by coughing (Table 3). These should therefore be consid-
6 months after initiation of indomethacin, however, re- ered in the differential diagnosis.
currences occurred in a few patients after a minimum
interval of 6 months [3,11].
The mechanism by which indomethacin is effective is Headache attributed to intracranial hypertension, idiopathic
not fully understood, but indomethacin decreases intra- or secondary
cranial pressure which could be the possible mechanism Headache attributed to intracranial hypertension, idio-
of action [32]. This could also explain why some studies pathic or secondary, is a non-pulsating headache which
found benefit in treating cough headache with acetazol- usually occurs daily and has a moderate intensity. It can
amide [25] and lumbar punctures [3,24], both known to worsen by coughing or other Valsalva manoeuvres. It is
decrease intracranial pressure. The latter even had a often accompanied with other abnormalities like
fairly good response rate with 8 out of 10 patients im- papilledema, visual field defects or a sixth nerve palsy
proved in the series published by Chen et al. [3]. [40,41].
Besides indomethacin, beneficial effects of topiramate
[33], methysergide [9,34], propranolol [35,36], naproxen Post-ictal headache
[37] and intravenous metoclopramide have been reported Post-ictal headache is a tension-type headache or, in a
[38] in smaller case series. patient with migraine, a migraine headache, appearing

Table 3 Primary and secondary headache disorders provoked or aggravated by coughing


Disorder Headache Cough as (Other) Cough as (Other) aggravators
quality trigger triggers aggravator
Primary cough headache sharp/stabbing ++ Valsalva NA NA
Symptomatic cough headache mixed nature ++ Valsalva NA NA
Idiopathic intracranial hypertension non-pulsating + Valsalva + Valsalva and postural changes
Headache attributed to intracranial non-pulsating + Valsalva + Valsalva
hypertension
Postictal headache pressing/ - seizure + bending and sudden head
pulsating movements
Headache attributed to Chiari mixed nature + Valsalva + NA
malformation
High altitude headache dull/pressing - > 2500 m + exertion, movement, straining and
bending
Migraine pulsating - see text + bending forward, exercise, …
Tension type headache pressing - see text + fatigue, stress, …
Cluster headache piercing + alcohol NA NA
NA: not applicable.
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after a partial or generalised epileptic seizure [42]. One present review describes the clinical characteristics and
study of 51 patients with post-ictal headache found that in treatment options of primary and symptomatic cough
half of the patients headache could be aggravated by headache. In addition, various headache disorders which
coughing, bending and sudden head movements [43]. can be aggravated or triggered by coughing were listed.
The present overview can guide clinicians in their diag-
High-altitude headache nostic and therapeutic process.
High-altitude headache can appear after an ascent to an
altitude above 2500 m. Typical features are onset within Competing interests
The authors declare that they have no competing interests.
24 hours of reaching a certain height with the appear-
ance of a usually bilateral and dull headache with a dur- Authors’ contributions
ation of less than one day [12,44]. Headache can be AC drafted the manuscript. WDH, JDK and JV provided essential comments
to finalize the manuscript. All authors read and approved the final
aggravated by exertion, movement, straining, coughing manuscript.
or bending [45].
Acknowledgements
Migraine Willem De Hertogh is partially supported by a research grant from the UZ
Brussel Willy Gepts Fund (2009).
Migraine is mostly a unilateral headache with a pulsating
quality. It is usually associated with nausea and/or photo- Author details
1
phobia and phonophobia. Physical activity is a well-known Headache Clinic, Department of Neurology, Universitair Ziekenhuis Brussel,
Brussel, Belgium. 2Department of Rehabilitation Sciences and Physiotherapy,
aggravating factor [46]. Spierings et al. investigated 38 pa- Faculty of Medicine and Health Sciences, University of Antwerp, Antwerp,
tients with migraine and reported that patients identified Belgium. 3Department of Neurology, University Medical Center Groningen,
Valsalva-related manoeuvres like straining (87%), bending Groningen, The Netherlands. 4Department of Neurology, Universitair
Ziekenhuis Brussel, Laarbeeklaan 101, 1090, Brussel, Belgium.
over (84%) and coughing/sneezing (53%) as aggravating or
triggering factors. Other reported triggers or aggravating Received: 11 February 2013 Accepted: 2 May 2013
factors were physical activity, stress, fatigue, reading, driv- Published: 20 May 2013
ing, lack of sleep, specific foods/drinks, alcohol, not eating
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Migralepsy, hemicrania epileptica, post-ictal headache and "ictal epileptic journal and benefit from:
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Headache Rep 11(4):293–296
7 High visibility within the field
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(2003) Clinical features of headache at altitude: a prospective study. 7 Retaining the copyright to your article
Neurology 60(7):1167–1171
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